Pediatric Duodenal Atresia and Stenosis Surgery Treatment & Management

The operative management of duodenal atresia is determined by the anatomic findings and associated anomalies noted upon laparotomy. Bypass procedures for duodenal atresia or stenosis include duodenoduodenostomy or duodenojejunostomy. Type 1 duodenal atresia can also be managed by simple duodenotomy with web excision.

Orogastric decompression of the stomach and fluid resuscitation should be promptly initiated. Orogastric losses are monitored and replaced. Broad-spectrum antibiotics and 1 mg vitamin K are administered. Parenteral nutrition is instituted on the first day of life via a peripherally inserted central catheter. When stable, the infant is taken to the operating room.

• Following precautions against hypothermia, the abdomen is entered via a transverse right upper quadrant incision. The stomach and first part of the duodenum are usually dilated and thickened and require decompression via the orogastric tube.
• The gallbladder and spleen are examined. The viscera are examined for other anomalies, including malrotation, an anterior portal vein, and an annular pancreas. The liver is padded and gently retracted superiorly.
• The duodenum is mobilized via Kocher maneuver.
• The site of obstruction is detected by the discrepancy in the size of the bowel above and below the obstruction and by passing the orogastric tube down to the level of the obstruction.
• In patients with a windsock web, an indentation at a site proximal to the site of obstruction may be observed. This marks the site of origin of the web and the location where the duodenotomy should be made.
• The papilla of Vater is identified following duodenotomy by gently pressing on the gallbladder and observing the proximal and distal segments. In patients with type 3 defects, dual biliary ducts may be present.
• Following documentation that no distal obstruction is present, proximal transverse to distal longitudinal (diamond-shaped) anastomosis may be performed using interrupted stitches (see following image). A direct duodenoduodenostomy is believed to result in a relatively earlier recovery of anastomotic function when compared with results following duodenojejunostomy.^[7] During the diamond-shaped anastomosis, the midpoint of the proximal incision is approximated to the end of the distal incision.^[4] This creates a larger stoma and allows the proximal duodenum to overlie the distal duodenum. As an alternative, a standard side-to-side duodenoduodenostomy may be performed. During the diamond-shaped anastomosis, a proximal transverse to distal longitudinal anastomosis is performed; the midpoint of the proximal incision is approximated to the end of the distal incision.
• Alternatively, type 1 defects may be treated by excising the web. An anterior duodenotomy is performed, and the web is opened along the lateral side. The web is carefully excised, leaving the medial portion containing the papilla in situ.
• When the proximal duodenum is floppy and dilated, an antimesenteric duodenoplasty may be performed either by excising excess tissue or by plicating the anterior wall with interrupted sutures over a dilator. This is believed to decrease the complications of a megaduodenum.^[20]
• If an annular pancreas is present, it should not be divided. The anastomosis is formed anterior to the pancreatic mass.
• In patients with malrotation, a Ladd procedure is performed; Ladd peritoneal bands are divided, the small bowel mesentery is widened, an appendectomy is performed, and the cecum and colon are placed on the left.
• Few case reports or case series look at short-term outcome for laparoscopic duodenoduodenostomy.^{[21, 22]} The basic principles described above for the open technique are carried out; however, this procedure requires advanced laparoscopic skills. This approach is facilitated by the use of 3-mm short instruments, better visualization at laparoscopy, use of Nitinol U-clips (Medtronic Surgical, Minneapolis, Minn), and the decompressed distal bowel associated with the atresia. Theoretically, assessment of the distal bowel for associated atresias is more difficult; however, there are no reports of missed distal atresias using the laparoscopic approach. Long-term outcomes and the ease with which other pediatric surgeons adopt this approach will determine how widely available laparoscopic duodenoduodenostomy becomes in the future.

The orogastric tube is left on free drainage. The patient is not given oral feedings until bowel sounds are heard, stool is passed, and the gastric drainage is limited (< 1 mL/kg/h of clear or pale-green fluid). This may take 7-10 days but can be prolonged in the premature infant with other significant anomalies that require venous access for parenteral nutrition.

Oral feedings are gradually introduced, commencing with clear fluids and aspirating the stomach prior to each feed. These infants can be placed right-side down after feeds to enhance gastroduodenal emptying.

Poor peristalsis in the proximal duodenum leads to functional obstruction in some patients. This may predispose the patient to blind loop syndrome and duodenogastric reflux. Alkaline biliary reflux leads to gastritis and peptic ulceration.

Reports of functional obstructions occurring more than 2 decades after primary repair suggest that survivors of duodenal atresia should receive the following long-term follow-up care:

• Clinical examination: Special attention is paid to the abdominal examination and the nutritional status of the patient.
• Radiologic examination: A barium meal may demonstrate the following:

  • Mildly dilated duodenum
  • Megaduodenum
  • Delayed emptying
  • Diminished peristalsis
  • Slight luminal narrowing
  • Bezoars
• Endoscopy: Findings on esophagogastroduodenoscopy include the following:

  • Esophageal irritation
  • Reflux gastritis
  • Megaduodenum
  • Delayed emptying

Early postoperative complications are frequently related to prematurity, coexisting congenital anomalies, and parenteral nutrition. Intestinal obstruction secondary to adhesions may also occur in the early postoperative period.

Long-term complications occur at any time from a few months to years following the primary procedure. Hence, long-term follow-up is compulsory for infants treated for intrinsic duodenal obstruction. Late complications include the following:^{[20, 23, 24]}

• Duodenal dysmotility
• Megaduodenum with blind loop syndrome
• Duodenogastric reflux and gastritis
• Peptic ulcer disease
• Gastroesophageal reflux
• Cholelithiasis
• Cholecystitis

Current survival rates for infants with duodenal atresia or stenosis are 90-95%. Higher mortality rates are associated with prematurity and multiple congenital abnormalities.

Postoperative complications are reported in 14-18% of patients; some require reoperation.^[2] Possible indications for reoperation include anastomotic leak, functional duodenal obstruction, adhesions, and missed atresias.

Long-term follow-up of these patients reveals that most of these patients are asymptomatic with a normal nutritional status. In a 1988 study, Kokkonen et al found a poor correlation between symptoms and radiologic and endoscopic findings; the megaduodenum failed to return to normal caliber, and duodenogastric reflux and duodenal dysmotility persisted decades after the initial surgery in asymptomatic patients.^[23] Approximately 12% of patients develop late complications. Late deaths occur in approximately 6% of patients, and 50% of these are related to complex cardiac conditions. Less than 10% of patients require fundoplication for gastroesophageal reflux, and less than 10% require revision of the initial repair.^[25]

Dysmotility disorders associated with megaduodenum can be managed with an antimesenteric tapering duodenoplasty or duodenal plication.

Duodenal webs can be diagnosed and excised by an expert surgical endoscopist. This is feasible in patients who present after the neonatal period with duodenal stenosis. The morbidity and complications associated with a megaduodenum may require further surgical intervention.

Transanastomotic feeding tubes or gastrostomies were used in the past but have been demonstrated to offer no clear advantage and, instead, result in a delay in establishing oral feedings and an increase in the duration of hospitalization.^{[26, 3, 27]}

Soutter and Askew (2003) reported successful results with a transumbilical approach to duodenal atresia.^[28]